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Peroxisomes, Myelination, and Axonal Integrity in the CNS

Laboratory of Cell Metabolism, KU Leuven, Leuven, Belgium, myriam.baes{at}pharm.kuleuven.be

Patrick Aubourg, MD

INSERM U745, Department of Pediatric Neurology, Hôpital Saint-Vincent de Paul, University René Descartes, Paris, France

Peroxisomes are ubiquitous organelles with multiple metabolic functions, but their precise role in the maintenance of tissues is not well understood. All diseases caused by partial or complete peroxisome dysfunction are characterized by a variety of neurological abnormalities, underscoring the importance of peroxisomes in nervous tissue. The interrelationship between metabolic abnormalities, histological changes, and clinical signs in these peroxisomal diseases has not yet been clarified. During the past decade, a more systematic study of the consequences of peroxisome dysfunction was possible through the generation of knockout mice with generalized or conditional inactivation of peroxisomal proteins. It appears that peroxisomes are necessary for the preservation of axonal integrity and for the formation and maintenance of myelin.

Key Words: peroxisomes • lipid metabolism • myelin • axon degeneration

The Neuroscientist, Vol. 15, No. 4, 367-379 (2009)
DOI: 10.1177/1073858409336297


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